Korean Circ J.  2006 Dec;36(12):820-822. 10.4070/kcj.2006.36.12.820.

A Case of Embolization Seen in Pulmonary Arteriovenous Malformation in a Patient with Osler-Rendu-Weber Syndrome

• Jang HJ
• Kim MS
• Kim SY
• Han JK
• Kim YJ
• Oh BH
• Jae HJ

• KMID: 2013166
• DOI: http://doi.org/10.4070/kcj.2006.36.12.820

Abstract

Osler-Rendu-Weber Syndrome or hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasia of the skin and of the mucous membranes and intermittent bleeding from vascular abnormalities; in about 20% of the patients pulmonary arteriovenous malformation is present. Pulmonary arteriovenous malformation is a congenital anomaly in the lung which causes shunting of venous blood in the pulmonary artery to systemic circulation, resulting in cyanosis, polycythemia and clubbing. Recently we experienced a case of multiple pulmonary arteriovenous malformation associated with the telangiectatic change of the cerebral artery in a 16-year-old male patient, which was confirmed by pulmonary angiography.

Keyword

Osler-Rendu-Weber syndrome; Arteriovenous malformation; Telangiectasia, hereditary hemorrhagic

MeSH Terms

Adolescent
Angiography
Arteriovenous Malformations*
Cerebral Arteries
Cyanosis
Hemorrhage
Humans
Lung
Male
Mucous Membrane
Polycythemia
Pulmonary Artery
Skin
Telangiectasia, Hereditary Hemorrhagic
Telangiectasis